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[外阴硬化性苔藓。早期临床及组织学诊断的重要性]

[Vulvar lichen sclerosus. The importance of early clinical and histological diagnosis].

作者信息

Regauer S, Liegl B, Reich O, Pickel H, Beham-Schmid C

机构信息

Institut für Pathologie, Arbeitsgruppen für Dermatopathologie, Gynäkopathologie, Hämatopathologie, Medizinische-Universität Graz.

出版信息

Hautarzt. 2004 Feb;55(2):158-64. doi: 10.1007/s00105-003-0645-8.

DOI:10.1007/s00105-003-0645-8
PMID:14968326
Abstract

Vulvar lichen sclerosus (LS) is a chronic progressive skin disease of unclear etiology. It is often overlooked in early stages, but progresses to destructive atrophy and is associated with an increased risk of vulvar squamous cell carcinoma. The classical symptoms are pruritus and pain, but they are often not distinctive, so that unclear vulvar problems often lead to a biopsy. The histological picture of early LS is quite different from that of late LS with an atrophic epidermis, markedly sclerotic dermis and stiff dilated vessels. The epidermis in early LS is usually normal with only minor irregularities in the rete pattern. The basement membrane is normal or focally widened, while the edematous dermis has only scattered ectatic vessels. The often dense lichenoid and intraepidermal infiltrate explains the spongiosis and vacuolization of the basal layer keratinocytes. Very early cases may only have a sparse lymphocytic infiltrate and hyper-/parakeratosis of the follicular ostia. Early topical therapy can dampen the progression to atrophic, irreversible LS.

摘要

外阴硬化性苔藓(LS)是一种病因不明的慢性进行性皮肤病。它在早期常被忽视,但会发展为破坏性萎缩,并与外阴鳞状细胞癌风险增加相关。典型症状是瘙痒和疼痛,但通常不具特异性,因此不明原因的外阴问题常导致活检。早期LS的组织学表现与晚期LS有很大不同,晚期表现为萎缩性表皮、明显硬化的真皮和僵硬扩张的血管。早期LS的表皮通常正常,仅 rete 模式有轻微不规则。基底膜正常或局灶性增宽,而水肿的真皮仅有散在的扩张血管。常密集的苔藓样和表皮内浸润解释了基底层角质形成细胞的海绵形成和空泡化。极早期病例可能仅有稀疏的淋巴细胞浸润和毛囊口的角化过度/角化不全。早期局部治疗可抑制向萎缩性、不可逆LS的进展。

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本文引用的文献

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Lichen sclerosus.硬化性苔藓
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Vulval punch biopsies: what is the experience of patients and do they alter management?外阴打孔活检:患者的体验如何,活检会改变治疗方式吗?
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Childhood vulvar lichen sclerosus: an increasingly common problem.儿童外阴硬化性苔藓:一个日益常见的问题。
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Does lichen sclerosus play a central role in the pathogenesis of human papillomavirus negative vulvar squamous cell carcinoma? The itch-scratch-lichen sclerosus hypothesis.硬化性苔藓在人乳头瘤病毒阴性的外阴鳞状细胞癌发病机制中起核心作用吗?瘙痒-搔抓-硬化性苔藓假说。
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10
Decrease in epidermal CD44 expression as a potential mechanism for abnormal hyaluronate accumulation in superficial dermis in lichen sclerosus et atrophicus.表皮CD44表达降低作为萎缩性硬化性苔藓真皮浅层透明质酸异常蓄积的潜在机制。
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